High expression of circulating vascular endothelial growth factor (VEGF) has been reported in patients with Kawasaki disease (KD). In the pathophysiology of KD, VEGF is considered to be involved, especially in the development of coronary artery lesions. This study aimed to examine whether the VEGF-634 promoter polymorphism is a marker of KD susceptibility or severity in Chinese patients in Taiwan. The study included 93 KD patients and 96 normal control subjects. Genotype and allelic frequencies for the VEGF gene polymorphism in the two groups were compared. The number of individuals with the VEGF-634 G/G genotype was significantly greater among the patients with KD than among the healthy control subjects (p = 0.011). The odds ratio for the development of KD in individuals with the VEGF-634 G/G genotype was found to be 2.03 (95% confidence interval [CI], 1.14–3.63) compared with the VEGF-634 G/C and VEGF-634 C/C genotypes. No significant difference was observed in the genotype or allelic frequencies of VEGF C-634 G polymorphism between the patients with and those without coronary artery lesions. In conclusion, the results suggest that VEGF-634 G/G genotype may be involved in the development of KD in Taiwanese children.